O-GlcNAcylation of the intellectual disability protein DDX3X exerts proteostatic cell cycle control

Open Biol. 2025 Jan;15(7):250064. doi: 10.1098/rsob.250064. Epub 2025 Jul 2.

Abstract

O-GlcNAcylation of intracellular proteins is a key regulator of diverse cellular and developmental processes. Previous studies have demonstrated the acute sensitivity of cell cycle progression to chemical and genetic manipulation of O-GlcNAc homeostasis. However, the mechanisms by which O-GlcNAc regulates the cell cycle remain poorly understood. Here, we report Ser584 O-GlcNAcylation of the RNA helicase DDX3X, a microcephaly associated protein, as a proteostatic mechanism regulating S-phase entry. Loss of Ser584 O-GlcNAcylation promoted degradation of DDX3X by the proteasome, resulting in reduced expression of the DDX3X target gene cyclin E1 and impaired cell cycle progression from G1 to S phase. These findings display how a single O-GlcNAc site affects DDX3X stability and thereby the cell cycle.

Keywords: O-GlcNAc; cell cycle; cell signalling.

MeSH terms

  • Acetylglucosamine* / metabolism
  • Cyclin E / genetics
  • Cyclin E / metabolism
  • DEAD-box RNA Helicases* / chemistry
  • DEAD-box RNA Helicases* / genetics
  • DEAD-box RNA Helicases* / metabolism
  • Glycosylation
  • HEK293 Cells
  • Humans
  • Intellectual Disability* / genetics
  • Intellectual Disability* / metabolism
  • Proteasome Endopeptidase Complex / metabolism
  • Protein Processing, Post-Translational
  • Proteolysis
  • Proteostasis*

Substances

  • DEAD-box RNA Helicases
  • DDX3X protein, human
  • Proteasome Endopeptidase Complex
  • Acetylglucosamine
  • Cyclin E